A 10 year old boy presented with multiple, red small raised lesions on the tongue for the last 4 years. The condition started as an asymptomatic single raised lesion on the undersurface of the tongue which gradually increased in number and extended onto the sides and upper surface of the tongue. There was no preceeding history of trauma or bleeding from lesions. Patient denied any history of similar lesions elsewhere on the body. His past medical history was unremarkable.

Fig.1 Raised lesions of angiokeratoma circumscriptum on the (a) dorsum of the tongue and (b) extending onto the ventral surface of the tongue.

On examination of the oral cavity, the patient was found to have two raised lesions of sizes 1×1cm and 2×3 cm, on the dorsum of the tongue, anterior to the base, which were extending onto the ventral surface of the tongue. They were studded with multiple grouped, erythematous shining papules some of which had a keratotic top. The masses were non-friable and moderately tender on palpation. They were mobile, firm on palpation, and did not bleed on manipulation. The rest of the cutaneous and systemic examination was normal. A biopsy specimen of a representative tongue lesion showed parakeratosis, acanthosis, papillomatosis with large dilated spaces lined by normal appearing endothelium and filled with erythrocytes and organizing thrombi. On the basis of clinical examination and histopathological findings, a diagnosis of angiokeratoma circumscriptum of the tongue was made.

Angiokeratoma circumscriptum is a rare vascular malformation of the papillary dermis manifesting as one or several purple papules and blood filled cystic nodules that gradually become verrucous and coalese into plaques. They may be linear or zosteriform pattern and bleed readily from trauma. Vessels are ectatic histologically and may be thrombosed. The overlying epidermis shows variable degree of hyperkeratosis, papillomatosis and acanthosis. The elongated rete ridges may partially or completely envelop the dilated vessels. Usually, the lesions are present at birth, but in some cases they may occur during childhood as in our case and even in adulthood. Angiokeratoma may be associated with Klippel-Trenaunay, Weber syndrome, Cobb syndrome and other mixed vascular malformations. The lesions are typically situated on the lower leg, foot, thigh or buttock but may occur elsewhere on the skin [5,6].

Oral involvement in angiokeratomas is most commonly a component of angiokeratoma corporis diffusum which is associated with several inherited lysosomal disorders [6] It is rare in other types of angiokeratomas.

Till date there are three reported cases of angiokeratoma circumscriptum solely localized to the oral cavity in pediatric patients [2-4]. Kumar, et al. reported the case of a 16 year old boy with histologically defined angiokeratoma circumscriptum on the ventral aspect of the tongue [3]. Another 12 year old boy with angiokeratoma circumscriptum isolated to the ventral tongue was reported [2]. The third patient was a 6-year old male who presented with a 2 year history of recurrent mass on the dorsal tongue [4]. Our case of lingual angiokeratoma circumscriptum involved both the dorsal and ventral surfaces of the tongue. Another interesting point to be noted is that all four patients including ours were male patients.

The pathogenesis of angiokeratomas is still unknown. It has been reported to develop overlying an arteriovenous fistula and in areas of lymphangioma circumscriptum after local injuries [7,8]. Angikeratomas may be treated with complete surgical excision, cryotherapy and laser ablation including copper vapour, potassium tritanyl phosphate, and argon lasers.

Contributors: All the authors have participated equally in the preparation of the article.

Funding: None; Competing interests: None stated.

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2. Vijaikumar M, Thappa DM, Karthikeyan K, Jayanthi S. Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol. 2003;20:180-2.

3. Kumar MV, Thappa DM, Shanmugam, Ratnakar C. Angiokeratoma circumscriptum of the oral cavity. Acta Derm Venereol. 1998;78:472.

4. Green JB, Roy S. Angiokeratoma circumscriptum of the dorsal tongue in a child. Int J Pediatr Dermatol. 2006;1:107-9.

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7. Foucar W, Nason WV. Angiokeratoma circumscriptum following damage to underlying vasculature. Arch Dermatol. 1986;122:245-6.